Abstract Introduction Interstitial lung disease (ILD) is conventionally thought of as a slowly progressive disease, being recognized after many consecutive medical visits. In contrast, RP-ILD often progresses considerably within weeks of symptom onset. In this report, we present a case of RP-ILD associated with Anti-MDA-5 dermatomyositis, a rare and underrecognized connective tissue disease where respiratory symptoms are often the first clue to a rheumatologic diagnosis. Case description A 44-year-old male with a history of e-cigarette smoking presented from an outside hospital with concern for worsening acute hypoxic respiratory failure. The patient had received empiric treatment for community acquired pneumonia and underwent further diagnostics which showed he was negative for COVID, Influenza, or HIV. He had no history of autoimmune or rheumatologic disease. The patient was initiated on intravenous methylprednisolone 80mg every 8 hours, in addition to antibiotics for empiric coverage of both PJP and MRSA. At our facility, the patient quickly escalated to requiring endotracheal intubation. High resolution CT chest showed a peripheral and basilar predominance of ground glass opacification with areas displaying central ground glass consolidation and peripheral opacification (Atoll sign.) Further history and diagnostics were obtained, which revealed the patient had been experiencing one month of lower extremity weakness. Physical examination revealed hyperkeratotic fissuring of the right hand, consistent with “mechanic’s hands.” The patient’s labs showed elevated CK, aldolase, and 1:320 ANA positivity. A broad antibody panel for dermatomyositis was ordered, with concern for anti-MDA-5 dermatomyositis. Given the known high mortality and rapid disease progression, we empirically began treatment with IVIG, JAK inhibitor, and pulse dose steroids. Due to further respiratory decompensation, the patient was placed on VV-ECMO and transferred for evaluation of lung transplantation. After four weeks, the antibodies for Anti-MDA-5 resulted as positive with high titers. The diagnosis of Rapidly Progressive Interstitial Lung Disease (RP-ILD) due to Anti-MDA-5 dermatomyositis was made. Discussion RP-ILD caused by Anti-MDA-5 dermatomyositis is an underrecognized clinical syndrome with high mortality due to poor corticosteroid response. The speed of pulmonary decompensation can pose challenges with the traditional time delays associated with obtaining diagnostics and coordinating multidisciplinary care. Anti-MDA-5 associated RP-ILD shows such a severe clinical course and poor response to traditional therapy that the ACR/CHEST 2023 guidelines recommend initial treatment include both IV steroids and two additional immune-compromising therapies. Our case shows the benefit of identifying this disease early to facilitate timely treatment and referral to a transplant-capable center. This abstract is funded by: None
Jha et al. (Fri,) studied this question.